First antibody that detects only known cause of Lou Gehrigs disease developed

Boffins at the University of Toronto have developed the first antibody that detects the only known cause of amyotrophic lateral sclerosis (ALS), also called Lou Gehrigs disease.

London, May 9 : Boffins at the University of Toronto have developed the first antibody that detects the only known cause of amyotrophic lateral sclerosis (ALS), also called Lou Gehrig's disease.

ALS is a disease marked by gradual degeneration of the nerve cells in the central nervous system that control voluntary muscle movement. The disorder causes muscle weakness and atrophy throughout the body. Eventually, the brain completely loses its ability to initiate and control voluntary movement.

Currently there is no test that can provide a definite diagnosis of ALS. Instead, the diagnosis of ALS is primarily based on the symptoms and signs a physician observes in the patient and a series of tests to rule out other diseases.

Professor Janice Robertson (Laboratory Medicine and Pathobiology) Canada Research Chair in the Molecular Mechanics of ALS at the Centre for Research in Neurodegenerative Diseases, and one of the lead authors of the study, said that the development of the antibody has researchers excited as it provides the first tool for recognizing misfolded conformations of the enzyme superoxide-dimutase-1 (SOD1).

SOD1 enzyme is a powerful antioxidant that protects the body from damage caused by superoxide, a toxic free radical. Free radicals are highly reactive molecules produced by cells during normal metabolism. Free radicals can accumulate and cause damage to DNA and proteins within cells.

"This antibody will enable researchers to investigate whether misfolded SOD1 is involved in other forms of ALS. This is important to determining if SOD1 is relevant in ALS cases that are not caused by mutations in SOD1. If this is the case, then the antibody could potentially be used in biomarker studies to facilitate earlier diagnosis of the disease," Nature quoted her, as saying.

Professor Avi Chakrabartty (Medical Biophysics and Biochemistry, Ontario Cancer Institute - University Health Network), senior author of the study, said that the antibody, named SOD1-exposed-dimer-interface antibody (SEDI-antibody), also opens up the possibility of developing immunization strategies for the treatment of ALS caused by SOD1 mutations.

"The SEDI antibody also has utility in drug discovery efforts for identifying chemical chaperones that prevent or reduce misfolding of SOD1 in ALS ," said Chakrabartty .

The study was published in the online edition on May 7th, and appears June print edition of Nature Medicine.

ANI